OBJECTIVES: To determine the causes of death and risk factors in systemic sclerosis (SSc). METHODS: Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. RESULTS: We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. CONCLUSION: Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.

Mapping and predicting mortality from systemic sclerosis / Elhai, Muriel; Meune, Christophe; Boubaya, Marouane; Avouac, Jã©rã´me; Hachulla, Eric; Balbir gurman, Alexandra; Riemekasten, Gabriela; Airã², Paolo; Joven, Beatriz; Vettori, Serena; Cozzi, Franco; Ullman, Susanne; Czirjã¡k, Lã¡szlã³; Tikly, Mohammed; Mã¼ller ladner, Ulf; Caramaschi, Paola; Distler, Oliver; Iannone, Florenzo; Ananieva, Lidia P; Hesselstrand, Roger; Becvar, Radim; Gabrielli, Armando; Damjanov, Nemanja; Salvador, Maria J; Riccieri, Valeria; Mihai, Carina; Szã¼cs, Gabriella; Walker, Ulrich A; Hunzelmann, Nicolas; Martinovic, Duska; Smith, Vanessa; Mã¼ller, Carolina De Souza; Montecucco, Carlo Maurizio; Opris, Daniela; Ingegnoli, Francesca; Vlachoyiannopoulos, Panayiotis G; Stamenkovic, Bojana; Rosato, Edoardo; Heitmann, Stefan; Distler, Jã¶rg H. W; Zenone, Thierry; Seidel, Matthias; Vacca, Alessandra; Langhe, Ellen De; Novak, Srdan; Cutolo, Maurizio; Mouthon, Luc; Henes, Jã¶rg; Chizzolini, Carlo; Mã¼hlen, Carlos Alberto Von; Solanki, Kamal; Rednic, Simona; Stamp, Lisa; Anic, Branimir; Santamaria, Vera Ortiz; Santis, Maria De; Yavuz, Sule; Sifuentes giraldo, Walter Alberto; Chatelus, Emmanuel; Stork, Jiri; Laar, Jacob Van; Loyo, Esthela; De La Peã±a Lefebvre, Paloma Garcia; Eyerich, Kilian; Cosentino, Vanesa; Alegre sancho, Juan Jose; Kowal bielecka, Otylia; Rey, Grã©goire; Matucci cerinic, Marco; Allanore, Yannick. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - STAMPA. - 76:11(2017), pp. 1897-1905. [10.1136/annrheumdis-2017-211448]

Mapping and predicting mortality from systemic sclerosis

RICCIERI, Valeria;ROSATO, Edoardo;
2017

Abstract

OBJECTIVES: To determine the causes of death and risk factors in systemic sclerosis (SSc). METHODS: Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. RESULTS: We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. CONCLUSION: Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.
2017
cardiovascular disease; epidemiology; pulmonary fibrosis; systemic sclerosis; Aged; Cause of Death; Databases, Factual; Death Certificates; Female; France; Humans; Male; Middle Aged; Prognosis; Proportional Hazards Models; Risk Factors; Scleroderma, Systemic; Time Factors; Rheumatology; Immunology and Allergy; Immunology; Biochemistry, Genetics and Molecular Biology (all)
01 Pubblicazione su rivista::01a Articolo in rivista
Mapping and predicting mortality from systemic sclerosis / Elhai, Muriel; Meune, Christophe; Boubaya, Marouane; Avouac, Jã©rã´me; Hachulla, Eric; Balbir gurman, Alexandra; Riemekasten, Gabriela; Airã², Paolo; Joven, Beatriz; Vettori, Serena; Cozzi, Franco; Ullman, Susanne; Czirjã¡k, Lã¡szlã³; Tikly, Mohammed; Mã¼ller ladner, Ulf; Caramaschi, Paola; Distler, Oliver; Iannone, Florenzo; Ananieva, Lidia P; Hesselstrand, Roger; Becvar, Radim; Gabrielli, Armando; Damjanov, Nemanja; Salvador, Maria J; Riccieri, Valeria; Mihai, Carina; Szã¼cs, Gabriella; Walker, Ulrich A; Hunzelmann, Nicolas; Martinovic, Duska; Smith, Vanessa; Mã¼ller, Carolina De Souza; Montecucco, Carlo Maurizio; Opris, Daniela; Ingegnoli, Francesca; Vlachoyiannopoulos, Panayiotis G; Stamenkovic, Bojana; Rosato, Edoardo; Heitmann, Stefan; Distler, Jã¶rg H. W; Zenone, Thierry; Seidel, Matthias; Vacca, Alessandra; Langhe, Ellen De; Novak, Srdan; Cutolo, Maurizio; Mouthon, Luc; Henes, Jã¶rg; Chizzolini, Carlo; Mã¼hlen, Carlos Alberto Von; Solanki, Kamal; Rednic, Simona; Stamp, Lisa; Anic, Branimir; Santamaria, Vera Ortiz; Santis, Maria De; Yavuz, Sule; Sifuentes giraldo, Walter Alberto; Chatelus, Emmanuel; Stork, Jiri; Laar, Jacob Van; Loyo, Esthela; De La Peã±a Lefebvre, Paloma Garcia; Eyerich, Kilian; Cosentino, Vanesa; Alegre sancho, Juan Jose; Kowal bielecka, Otylia; Rey, Grã©goire; Matucci cerinic, Marco; Allanore, Yannick. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - STAMPA. - 76:11(2017), pp. 1897-1905. [10.1136/annrheumdis-2017-211448]
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